Chronic neutropenia: how best to assess severity and approach management?

ABSTRACT

Introduction

Neutropenia is a relatively common finding in medical practice and the medical approach requires a gradual and pertinent diagnostic procedure as well as adapted management.

Areas Covered

The area of chronic neutropenia remains fragmented between diverse diseases or situations. Here physicians involved in different aspects of chronic neutropenia gather both the data from medical literature till the end of May 2021 and their experience to offer a global approach for the diagnosis of chronic neutropenia as well as their medical care.

Expert opinion

In most cases, the neutropenia is transient, frequently related to a viral infection, and not harmful. However, neutropenia can be chronic (i.e. >3 months) and related to a number of etiologies, some clinically benign, such as so-called ‘ethnic’ neutropenia. Autoimmune neutropenia is the common form in young children, whereas idiopathic/immune neutropenia is a frequent etiology in young females. Inherited neutropenia (or congenital neutropenia) is exceptional, with approximately 30 new cases per 10⁶ births and 30 known subtypes. Such patients have a high risk of invasive bacterial infections, and oral infections. Supportive therapy, which is primarily based on daily administration of an antibiotic prophylaxis and/or treatment with granulocyte-colony stimulating factor (G-CSF), contributes to avoiding recurrent infections.

KEYWORDS:

• Neutropenia
• children
• gcsf
• diagnostic evaluation
• classification

Article highlights

• There is much information available in the literature concerning neutropenia related to chemotherapy, but very little regarding chronic neutropenia even though management is very different than chemotherapy induced neutropenia.

• Chronic neutropenia is a very common finding, as blood count is now a very common clinical test

• The first step of the medical management of a chronic neutropenia is a specific diagnosis. The most frequent cause of chronic neutropenia is chronic benign neutropenia, frequently associated with the Duffy Null phenotype most commonly seen in those of African ancestry.

• Other benign causes of neutropenia are post viral neutropenia, auto immune neutropenia in young children, idiopathic/immune neutropenia in young adults.

• Congenital neutropenia or inherited neutropenia –i.e. chronic neutropenia related to monogenic forms of neutropenia – is a very rare cause of chronic neutropenia, usually diagnosed because it is associated with severe infections, recurrent oral infections and associated organ dysfunction.

• Medical care should be adapted to the clinical severity of the neutropenia, some benign situations necessiting no medical intervention, while some other situations necessitate medical intervention, ranging from prophylactic antibiotic therapy, G-CSF therapy or definitive cure by allogeneic hematopoietic stem cell transplantation.

Declaration of interest

J Donadieu declared consultancy honorarium with X4 Pharma in 2018. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.